Objective:To summarize the clinical characteristics and surgical outcomes of endolymphatic sac tumor（ELST）, and improve the experience of diagnosis and treatment of this disease. Methods:A retrospective analysis was conducted on the clinical data of patients with ELST who underwent surgical treatment by the Department of Otolaryngology Head and Neck Surgery, Beijing Friendship Hospital, Capital Medical University from January 2015 to December 2024.The clinical and image features, perioperative management, surgical methods and follow-up results of the disease were summarized. Results:Of the 6 cases, 4 were male and 2 were female. The primary clinical characteristics were hearing loss（6 cases）, tinnitus（5 cases）, dizziness（2 cases）, facial paralysis（1 case）, and headache（1 case）. CT and MRI of temporal bone were performed in all cases. The manifestation of CT was a space occupying lesion centered on the region of endolymphatic sac, accompanied by bone destruction and intertumoral calcification. MRI showed tumor center isosignal and peripheral hypersignal in T1 and T2 sequences in 3 cases, and mixed hypersignal in T1 and T2 sequences in 3 cases. Enhancement was observed in all cases on the enhanced scan. 5 cases underwent DSA examination and showed the tumors were supplied by the occipital artery（2 cases）, posterior auricular artery（4 cases）, and the bunch of internal carotid artery（1 case）. Embolization of the feeding artery was performed in 3 patients. Five patients underwent tumor resection. Translabyrinthine approach were performed in 4 cases, and middle cranial fossa approach was performed in 1 case. All cases followed up for 24 to 70 months with no distant metastases or death. Two patients experienced twice recurrences and were treated with surgical operation. The tumors were found to be closely related to the internal auditory canal or dura during the surgery. Conclusion:The clinical manifestations of ELST are not typical, and hearing loss is the most common clinical symptom. CT and MRI exhibit typical imaging characteristics. ELST has a risk of recurrence, and the tumor should be carefully managed when invade to the internal auditory canal and dura to reduce postoperative recurrence.
Humans ; Endolymphatic Sac/surgery* ; Male ; Female ; Retrospective Studies ; Ear Neoplasms/diagnosis* ; Magnetic Resonance Imaging ; Tomography, X-Ray Computed ; Adult ; Middle Aged ; Treatment Outcome

Humans ; Facial Nerve/surgery* ; Endolymphatic Sac/surgery* ; Cranial Sinuses ; Postoperative Complications ; Neoplasms

A 27-year-old female patient suffering endolymphatic sac tumor with intralabyrinthine hemorrhage was reported. The patient had hearing loss in the left ear with continuous tinnitus, and MRI showed the soft tissue shadow of endolymphatic sac. Considering that the tumor involved semicircular canal and vestibule,endolymphatic cyst tumor resection was performed by labyrinth route. After surgery, there was no cerebrospinal fluid leakage and facial nerve function was normal. More importantly, enhanced MRI of temporal bone showed no tumor recurrence 1 year after surgery.
Female ; Humans ; Adult ; Endolymphatic Sac/surgery* ; Neoplasm Recurrence, Local/pathology* ; Labyrinth Diseases ; Tinnitus ; Ear Neoplasms/pathology* ; Bone Neoplasms ; Hemorrhage

To investigate the postoperative anxiety and its relationship with life quality in patients with Ménière's diseases.
 Methods: A total of 68 patients with Ménière's disease, who received the treatment of endolymphatic sac decompression from 2010 to 2016, were enrolled. They finished two scales (the self-rating anxiety scale and the quality of life questionnaire for endolymphatic sac decompression of Ménière's disease) by snail mail. The patients were divided into different groups based on their preoperative course, postoperative follow-up time, clinic stage and quality of life after endolymphatic sac decompression surgery, and their anxiety was analyzed. In addition, 109 gender and age-matched patients with other diseases of otorhinolaryngology served as the control group, and their anxiety was also analyzed.
 Results: There was no statistical difference in the postoperative anxiety among the patients with preoperative course for less than 1 year, 1 to 5 years or more than 5 years (all P>0.05). There was no statistical difference in the patients with different duration of follow-up (P>0.05). There was no statistical difference in the patients with the different clinic stages (all P>0.05). There was no statistical difference in the patients with the improved life or non-improved life after surgery (P>0.05). The degree of the anxiety in the Ménière's group was more severe than that in the control group (P<0.01).
 Conclusion: It needs to keep in mind that a certain degree of postoperative anxiety still keeps in patients with Ménière's disease.
Anxiety ; Anxiety Disorders ; Case-Control Studies ; Decompression, Surgical ; methods ; Endolymphatic Sac ; surgery ; Humans ; Meniere Disease ; psychology ; surgery ; Postoperative Complications ; psychology ; Quality of Life

The purpose of the study was to observe changes in endolymphatic hydrops by using intratympanic injection of gadolinium and magnetic resonance imaging (MRI) before and after endolymphatic sac surgery in patients with unilateral Meniere's disease. Thirteen patients with unilateral Meniere's disease undergoing endolymphatic sac surgery were retrospectively and prospectively analyzed. Three-dimensional fluid-attenuated inversion recovery or three-dimensional real inversion recovery MRI was performed 24 h after an intratympanic injection of gadolinium to grade the presence of endolymphatic hydrops. Among the 13 patients with hydrops confirmed by preoperative MRI, vestibular hydrops had no significant change in all patients; cochlear hydrops became negative in 2 patients, and remained unchanged in the other 11 patients after surgery. Definite vertigo attacks were substantially controlled in one patient and completely controlled in 12 patients during a follow-up period of 8-34 months after surgery. The hearing levels were improved in 3 patients, remained unchanged in 7 patients, and decreased in 3 patients. In conclusion, endolymphatic sac surgery does not always alleviate endolymphatic hydrops in patients with Meniere's disease. Relief from vertigo cannot always be attributed to the remission of hydrops. A change in hearing levels cannot be explained by hydrops status alone.
Adult ; Aged ; Contrast Media ; administration & dosage ; Endolymphatic Hydrops ; diagnostic imaging ; pathology ; surgery ; Endolymphatic Sac ; diagnostic imaging ; pathology ; surgery ; Female ; Gadolinium ; administration & dosage ; Humans ; Imaging, Three-Dimensional ; Magnetic Resonance Imaging ; Male ; Meniere Disease ; diagnostic imaging ; pathology ; surgery ; Middle Aged

OBJECTIVE: To compare the vertigo controlling situation between the endolymphatic sac decompression(ELSD) and semicircular canal occlusion (SCO) in stage 4 Ménière disease. METHOD: Fourteen patients who underwent endolymphatic sac decompression and 9 patients who underwent semicircular canal occlusion from 2009 to 2013 were followed. All patients has complete preoperative examination and postoperative follow-up. RESULT: The vetigo control of the patients underwent endolymphatic sac decompression: completely control 35.7%; basic control 28.6%; partly control 14.3%; the vetigo control of the patients underwent semicircular canal occlusion: completely control 88.9%; basic control 11.1%. CONCLUSION ELSD and SCO are alternative methods for the patients of stage 4. SCO has a much highter complete vertigo control rate. For the patients without practical listening, SCO is a better choice.
Endolymphatic Sac ; surgery ; Humans ; Meniere Disease ; surgery ; Otologic Surgical Procedures ; methods ; Semicircular Canals ; surgery ; Vertigo ; surgery

This paper aims to identify emerging evidence for endolymphatic sac surgery (ESS) in the treatment of Meniere's disease since the landmark study by Thomsen et al, published in 1998 (conducted from 1981 to 1989). Using the MEDLINE database (PubMed), a systematic review of the literature published from January 1990 to June 2014 was performed. We included all English-language, peer-reviewed randomised controlled trials (RCTs) and controlled studies. Single-arm cohort studies were included if the sample size was ≥ 90 with a response rate > 60%. Altogether, 11 studies fulfilled our inclusion criteria; one was an RCT, two were controlled trials and eight were single-arm cohort studies. There currently exists a low level of evidence for the use of ESS in the treatment of Meniere's disease. Further studies, in particular RCTs and/or controlled studies, are required to fully evaluate this modality. However, there are difficulties in designing a valid placebo and achieving adequate blinding of observers and investigators.
Endolymphatic Sac ; surgery ; Humans ; Meniere Disease ; surgery ; Otologic Surgical Procedures ; methods

OBJECTIVETo study the clinicopathologic features and immunophenotype of endolymphatic sac tumor (ELST) and normal endolymphatic sac.

METHODSThe clinical and histologic features were evaluated in 5 cases of ELST. Eight cases of choroid plexus papilloma at cerebellopontine angle and 2 cases of normal endolymphatic sac were used as controls. Immunohistochemical study for vimentin, AE1/AE3, CK8/18, CK5/6, EMA, GFAP, synaptophysin, S-100 protein, CEA, TTF-1, VEGF, D2-40, calponin, calretinin and Ki-67 was carried out.

RESULTSThe age of onset of ELST ranged from 23 to 35 years (median = 24 years). The male-to-female ratio was 2:3. The clinical presentation was tinnitus, otalgia, hearing loss, otorrhagia with effusion and headache. The duration of symptoms ranged from 6 months to 10 years. Local recurrences were noted in 3 cases. Radiologically, the tumors were located at cerebellopontine angle and demonstrated petrous bone destruction. Histologic examination showed that the tumors had a papillary-glandular pattern. The papillae were covered by a single layer of low cuboidal cells. The tumor cells had distinct cell borders and contained eosinophilic to clear cytoplasm. The nuclei were slightly atypical and sometimes apically located. Focal dilated glandular structures with colloid-like material were also identified. The surrounding stroma was vascularized. All of the 5 cases had dural or petrous bone infiltration. Immunohistochemical study showed that all of the 5 cases were positive for AE1/AE3, CK8/18, CK5/6 and VEGF, 4 cases for EMA, 3 cases for calponin (focal), 2 cases for vimentin, 2 cases for S-100 protein, 1 case for GFAP and 1 case for synaptophysin (focal and weak). The Ki-67 index measured less than 1%. The staining for D2-40, calretinin, CEA and TTF-1 was negative. The 2 cases of the normal endolymphatic sac were positive for AE1/AE3 and CK8/18, and negative for CK5/6, EMA, S-100 protein, GFAP and synaptophysin. The 8 cases of choroid plexus papilloma were positive for synaptophysin. Seven cases were also positive for S-100 protein, 2 cases for GFAP and 1 case for D2-40. All of the 8 cases were negative for EMA, CK5/6 and calponin.

CONCLUSIONSELST is a rare slow-growing and potentially malignant tumor with a tendency of bone invasion and local recurrence. Distant metastasis is not observed. It must be distinguished from choroid plexus papilloma occurring at cerebellopontine angle. Correlation with clinical, radiologic and immunohistochemical findings would also be helpful.

Adenocarcinoma ; diagnostic imaging ; metabolism ; pathology ; surgery ; Adult ; Calcium-Binding Proteins ; metabolism ; Cerebellar Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cerebellopontine Angle ; pathology ; Diagnosis, Differential ; Endolymphatic Sac ; pathology ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Keratin-5 ; metabolism ; Keratin-6 ; metabolism ; Male ; Microfilament Proteins ; metabolism ; Mucin-1 ; metabolism ; Neoplasm Recurrence, Local ; Papilloma, Choroid Plexus ; metabolism ; pathology ; Tomography, X-Ray Computed ; Young Adult

Endolymphatic sac papillary tumor (ELST) is an extremely rare and aggressive tumor characterized by hearing loss and temporal bone destruction. A case with clinical, imaging, pathologic and treatment data is reported and relevant literature is reviewed. A 25-year-old woman, with ELST underwent craniotomy for tumor subtotal resection, and the diagnosis was confirmed by pathologic examination. Postoperative radiotherapy consisted of 50.4 Gy/28 f was given accordingly. The patient is currently alive with no signs of tumor recurrence locally and no radiation side-effects observed after one year follow-up. Complete resection is impossible in most cases, local resection, adjuvant radiotherapy may provide favored local control. A long-term follow-up is highly advocated in consideration of its slow development course.
Adult ; Ear Neoplasms ; diagnostic imaging ; pathology ; surgery ; Endolymphatic Sac ; diagnostic imaging ; pathology ; surgery ; Female ; Humans ; Radiography

Adenoma ; metabolism ; pathology ; Adult ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Ear Neoplasms ; complications ; genetics ; metabolism ; pathology ; surgery ; Endolymphatic Sac ; Female ; Follow-Up Studies ; Humans ; Kidney Neoplasms ; metabolism ; pathology ; Middle Aged ; Paraganglioma ; metabolism ; pathology ; Point Mutation ; Von Hippel-Lindau Tumor Suppressor Protein ; genetics ; von Hippel-Lindau Disease ; complications ; genetics ; metabolism